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GAA Recombinant Rabbit Monoclonal Antibody [JE54-59]

Usd: 385

Specification

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Catalog# ET7110-77

GAA Recombinant Rabbit Monoclonal Antibody [JE54-59]

Application

  • WB

  • IF-Tissue

  • IHC-P

Reactivity

  • Human

Conjugation

  • unconjugated

This product has been cited in peer reviewed publications, see list HERE

Overview

Product Name

GAA Recombinant Rabbit Monoclonal Antibody [JE54-59]

Antibody Type

Recombinant Rabbit monoclonal Antibody

Immunogen

Recombinant protein within Human GAA aa 120-230 / 952.

Species Reactivity

Human

Validated Applications

WB, IF-Tissue, IHC-P

Target Molecular Weight

Predicted band size: 105/76/70 kDa

Positive Control

HepG2 cell lysate, MCF-7 cell lysate, human placenta tissue lysate, human liver tissue, human placenta tissue, human liver tissue, human liver carcinoma tissue, HepG2.

Conjugation

unconjugated

Clone Number

JE54-59

RRID

AB_3071038

Product Features

Form

Liquid

Concentration

1 mg/mL.(The concentration of this product may be batch-dependent)

Storage Instructions

Shipped at 4℃. Store at +4℃ short term (1-2 weeks). Store at -20℃ long term.

Storage Buffer

1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.

Isotype

IgG

Purification Method

Protein A affinity purified.

Application Dilution

  • WB

  • 1:500-1:2,000

  • IF-Tissue

  • 1:50-1:200

  • IHC-P

  • 1:50-1:1,000

Target

Function

This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants.

Background References

1. Ostojic SM. et. al. Serum GAA as a Possible Biomarker of Exhaustive Exercise? Front Physiol. 2019 Dec.

2. Luo X. et. al. FISH analysis of Zanthoxylum armatum based on oligonucleotides for 5S rDNA and (GAA)(6). Genome. 2018 Sep.

Sequence Similarity

Belongs to the glycosyl hydrolase 31 family.

Post-translational Modification

The different forms of acid glucosidase are obtained by proteolytic processing.; Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor.

Subcellular Location

Lysosome, lysosome membrane.

UNIPROT

SwissProt: P10253 Human

Synonyms

70 kDa lysosomal alpha-glucosidase antibody

Acid alpha glucosidase antibody

Acid maltase antibody

Aglucosidase alfa antibody

Alpha glucosidase antibody

GAA antibody

Glucosidase alpha acid (Pompe disease glycogen storage disease type II) antibody

Glucosidase alpha acid antibody

Glucosidase alpha antibody

LYAG antibody

Expand

Images

  • Western blot analysis of GAA on different lysates with Rabbit anti-GAA antibody (<a href="/products/ET7110-77" style="font-weight: bold;text-decoration: underline;">ET7110-77</a>) at 1/2,000 dilution.<br /><br />Lane 1: HepG2 cell lysate<br />Lane 2: MCF7 cell lysate<br /><br />Lysates/proteins at 20 µg/Lane.<br /><br />Predicted band size: 105 kDa<br />Observed band size: 110,76,95 kDa<br /><br />Exposure time: 6 seconds;<br /><br />4-20% SDS-PAGE gel.<br /><br />Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (<a href="/products/ET7110-77" style="font-weight: bold;text-decoration: underline;">ET7110-77</a>) at 1/2,000 dilution was used in 5% NFDM/TBST at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody (<a href="/products/HA1001" style="font-weight: bold;text-decoration: underline;">HA1001</a>) at 1/50,000 dilution was used for 1 hour at room temperature.

    Western blot analysis of GAA on different lysates with Rabbit anti-GAA antibody (ET7110-77) at 1/2,000 dilution.

    Lane 1: HepG2 cell lysate
    Lane 2: MCF7 cell lysate

    Lysates/proteins at 20 µg/Lane.

    Predicted band size: 105 kDa
    Observed band size: 110,76,95 kDa

    Exposure time: 6 seconds;

    4-20% SDS-PAGE gel.

    Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (ET7110-77) at 1/2,000 dilution was used in 5% NFDM/TBST at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1/50,000 dilution was used for 1 hour at room temperature.

  • <span style="font-weight: bold;">☑ Knockdown (KD)</span><br /><br />Western blot analysis of GAA on different lysates with Rabbit anti-GAA antibody (<a href="/products/ET7110-77" style="font-weight: bold;text-decoration: underline;">ET7110-77</a>) at 1/1,000 dilution.<br /><br />Lane 1: HCT 116-si NT cell lysate<br />Lane 2: HCT 116-si GAA cell lysate<br /><br />Lysates/proteins at 10 µg/Lane.<br /><br />Predicted band size: 105 kDa<br />Observed band size: 75-110 kDa<br /><br />Exposure time: 10 seconds; ECL: K1801;<br /><br />4-20% SDS-PAGE gel.<br /><br />Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (<a href="/products/ET7110-77" style="font-weight: bold;text-decoration: underline;">ET7110-77</a>) at 1/1,000 dilution was used in 5% NFDM/TBST at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody (<a href="/products/HA1001" style="font-weight: bold;text-decoration: underline;">HA1001</a>) at 1/50,000 dilution was used for 1 hour at room temperature.

    ☑ Knockdown (KD)

    Western blot analysis of GAA on different lysates with Rabbit anti-GAA antibody (ET7110-77) at 1/1,000 dilution.

    Lane 1: HCT 116-si NT cell lysate
    Lane 2: HCT 116-si GAA cell lysate

    Lysates/proteins at 10 µg/Lane.

    Predicted band size: 105 kDa
    Observed band size: 75-110 kDa

    Exposure time: 10 seconds; ECL: K1801;

    4-20% SDS-PAGE gel.

    Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (ET7110-77) at 1/1,000 dilution was used in 5% NFDM/TBST at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1/50,000 dilution was used for 1 hour at room temperature.

  • Immunohistochemical analysis of paraffin-embedded human liver carcinoma tissue with Rabbit anti-GAA antibody (<a href="/products/ET7110-77" style="font-weight: bold;text-decoration: underline;">ET7110-77</a>) at 1/200 dilution.<br /><br />The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH<sub>2</sub>O and PBS, and then probed with the primary antibody (<a href="/products/ET7110-77" style="font-weight: bold;text-decoration: underline;">ET7110-77</a>) at 1/200 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.

    Immunohistochemical analysis of paraffin-embedded human liver carcinoma tissue with Rabbit anti-GAA antibody (ET7110-77) at 1/200 dilution.

    The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET7110-77) at 1/200 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.

  • Immunohistochemical analysis of paraffin-embedded human liver tissue with Rabbit anti-GAA antibody (<a href="/products/ET7110-77" style="font-weight: bold;text-decoration: underline;">ET7110-77</a>) at 1/1,000 dilution.<br /><br />The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH<sub>2</sub>O and PBS, and then probed with the primary antibody (<a href="/products/ET7110-77" style="font-weight: bold;text-decoration: underline;">ET7110-77</a>) at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.

    Immunohistochemical analysis of paraffin-embedded human liver tissue with Rabbit anti-GAA antibody (ET7110-77) at 1/1,000 dilution.

    The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET7110-77) at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.

  • Immunohistochemical analysis of paraffin-embedded human placenta tissue with Rabbit anti-GAA antibody (<a href="/products/ET7110-77" style="font-weight: bold;text-decoration: underline;">ET7110-77</a>) at 1/1,000 dilution.<br /><br />The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH<sub>2</sub>O and PBS, and then probed with the primary antibody (<a href="/products/ET7110-77" style="font-weight: bold;text-decoration: underline;">ET7110-77</a>) at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.

    Immunohistochemical analysis of paraffin-embedded human placenta tissue with Rabbit anti-GAA antibody (ET7110-77) at 1/1,000 dilution.

    The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (ET7110-77) at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX.

  • Flow cytometric analysis of GAA was done on HepG2 cells. The cells were fixed, permeabilized and stained with the primary antibody (<a href="/products/ET7110-77" style="font-weight: bold;text-decoration: underline;">ET7110-77</a>, 1/50) (red). After incubation of the primary antibody at room temperature for an hour, the cells were stained with a Alexa Fluor 488-conjugated Goat anti-Rabbit IgG Secondary antibody at 1/1000 dilution for 30 minutes.Unlabelled sample was used as a control (cells without incubation with primary antibody; black).

    Flow cytometric analysis of GAA was done on HepG2 cells. The cells were fixed, permeabilized and stained with the primary antibody (ET7110-77, 1/50) (red). After incubation of the primary antibody at room temperature for an hour, the cells were stained with a Alexa Fluor 488-conjugated Goat anti-Rabbit IgG Secondary antibody at 1/1000 dilution for 30 minutes.Unlabelled sample was used as a control (cells without incubation with primary antibody; black).

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